Lipoma and agenesis of the corpus callosum with associated choroid plexus lipomas. In utero diagnosis.

Abstract

Lipoma of the corpus callosum is a rare but well-de· scribed entity in the radiologic and neurosurgical literature. It is Jess well-appreciated that choroid plexus lipomas are associated lesions. Seven cases of corpus callosal lipomas diagnosed with ultrasound in the neo· natal period have been reported. We report its identification in utero, associated with choroid plexus lipomas and agenesis of the corpus callosum. CASE REPORTSCase 1 A 20-year-old (gravida 1 para 0) white female was referred at 37 5 weeks gestation with a question of fetal hydro~ cephalus noted on a study done to evaluate fetal presentation. An outside ultrasound had been done at 16 weeks to establish dates. This was unavailable for comparison.There was a single fetus in a breech position. Size was con• sistent with dates. The head circumference was at the 90th percentile. The trigone and occipital horns of the lateral ventricles were enlarged. The temporal and frontal horns rapidly returned to normal size, suggesting a dysplastic enlargement or colpocephaly. A brightly echogenic mass was present in the area of the anterior genu of the corpus callosum. The perical• losal and callosal marginal arteries were beating within the Received December 20, 1988, mass and not displaced by it. Symmetric masses were also present along the anterior portion of the lateral ventricle cho~ roid plexus. These were direct extensions from the central mass. The corpus callosum was absent. The medial sulci had a radial arrangement posterior to the central mass. The frontal horns were widely spaced (Fig. 1). These findings are typical for a lipoma of the corpus callosum with associated choroid plexus lipomas and agenesis of the corpus callosum. No other abnormalities were identified.The fetus remained breech and was delivered by cesarean section at term. The Apgar scores were 3 and 8 with normal umbilical cord gases. The neurologic examination was normal. The head circumference was at the 90%. but had a normal growth rate on follow -up evaluation. The neonatal head ultra· sound and computed tomography (CT) confirmed the findings with two additional points. There was a small lipoma in the left sylvian fissure not identified in utero. The CT showed extensive calcifications around the margin of the central mass. These were not visualized in utero and were very difficult to see on the neonatal ultrasound (Fig. 2).Case 2 An ultrasound examination was performed on a 33-year·old (gravida 3, para 1, abortus 1) white female for newly diagnosed gestational diabetes at 35 weeks gestation. A prior study had been done at 11 weeks for dating and question of viability. The intracranial contents on review, including videotape, were normal.The fetus was in a deep vertex lie with a calculated weight greater than the 90th percentile. A hyperechoic mass was identified in the anterior interhemispheric fissure typical for a lipoma (Fig. 3). Because of body habitus and fetal lie, a detailed intracranial evaluation was difficult to perform. The ventricles were normal in size. The corpus ...

Prenatal sonographic diagnosis of a pontine lipoma

“…In two of these instances the lesions were located above the tentorium and in the midline and were associated with complete agenesis of the corpus callosum. 6 One of these cases was also associ~ ated with bilateral lateral ventricular choroid plexus lipomas. Yock stated that as many as 20 to 25% of callosal lipomas are associated with choroid plexus lipomas.…”

Prenatal and Postnatal Imaging of Multiple Intracranial Lipomas: Report of a Case

“…They are commonly described as bright echogenic masses, whose typical location is at the level of the pericallosal cistern. Location at the level of the choroid plexus has only rarely been described [2,4] . Pericallosal lipomas may interfere with the normal growth of the corpus callosum, and as a consequence callosal agenesis or hypoplasia is commonly associated.…”

“…Pericallosal lipomas may interfere with the normal growth of the corpus callosum, and as a consequence callosal agenesis or hypoplasia is commonly associated. Additional anomalies may be encountered in these fetuses, usually midline defects (cleft lip or palate, median cleft nose, hypertelorism, or cerebellar vermis defects) or dysraphisms (myelomeningocele, encephalocele, anomalies of the cervical spine, cranium bifidum or other calvarial defects) [1,4] . On the other hand, due to the paucity of data it is uncertain if having multiple, rather than a single, intracranial lipomas is expected to have different prognostic implications.…”

“…In a fetus with a prenatally diagnosed intracranial lipoma, prognosis depends mostly on the presence and the type of associated anomalies [1,4] . In particular, it seems that among fetuses with intracranial lipoma and callosal agenesis, perinatal outcome is not influenced by the presence of the lipoma itself [1] .…”

“…Multiple intracranial lipomas have been almost exclusively reported in postnatal pediatric series [3,5] with only one case being sonographically diagnosed in utero to date [4] .We herein report a case of a fetus with multiple intracranial lipomas associated to agenesis of the corpus callosum diagnosed in utero by prenatal ultrasound and MRI findings. …”